Management

Management of CF

The bedrock of pulmonary management is: 1) the aggressive use of oral, intravenous and nebulised antibiotics to prevent and treat infection; 2) airway clearance, including exercise and the choice of a number of physiotherapy techniques; 3) avoidance of active and passive smoking; and 4) full immunisation, including influenza annually. Mucoactive agents employed include rhDNAse, hypertonic saline and mannitol. Macrolide antibiotics have been shown to be beneficial, although the exact mechanism has not been determined. Other anti-inflammatory drugs are more controversial. Pancreatic insufficiency is treated with pancreatic enzyme replacement therapy, sometimes supplemented by gastric acid-lowering strategies such as H2 receptor antagonists and proton pump inhibitors; DIOS may require gastrograffin orally or by enema, or intestinal lavage with Klean-Prep (Helsinn). CF-related diabetes is treated with insulin. The reader is referred to standard texts for more detailed discussions of treatment options for the less usual complications.

Patterns of care

Care in a specialist CF centre is essential. Definitions of what constitutes a ‘centre’ are neither evidence based nor uniformly agreed. The definition will be modified according to local needs; what is feasible in a densely populated urban area will be impracticable in a country with a very dispersed population. In such cases, core expertise may need to be collected in a central location, supporting more distant centres with clinic visits, telemedicine, and the implementation of agreed treatment protocols. The ideal CF centre should include the following:

A critical mass of patients sufficient to maintain expertise; ideally a minimum of 100 paediatric or adult patients, though this may not be possible in many parts of Europe.

A core multidisciplinary team (MDT) of CF specialist health professionals, or, in satellite centres, professionals who among their other commitments will ensure that they maintain competence in CF, with particular attention to continuing professional development. This group will comprise: at least two appropriately trained specialist paediatricians or adult physicians; clinical nurse specialists; physiotherapist; dieticians; a social worker; a psychologist; a pharmacist; and administrative support.

Enough personnel for cross cover during periods of annual and study leave.

Other medical and paramedical staff with experience of CF; for example, ward nurses who understand the requirements of the condition.

Expertise throughout the MDT in the complications of CF and the specialised procedures required by people with CF, or at least access to a major centre in the country that is able to manage these.

Support of staff from other specialist services with experience in management of the related issues that arise in patients with CF, such as ear, nose and throat specialist expertise, endocrinology, obstetrics and surgery.

Access to diagnostic and specialist laboratory facilities, particularly microbiology. Some services (genetics, for example) may be provided off-site at a national referral centre.

Facilities for inpatient and outpatient treatment, including an appropriate number of beds for people with CF.

Regular audit of practice.

Guidelines for management have been published, and the reader is referred to the websites of the ECFS (www.ecfs.eu), the UK CF Trust (www.cftrust.org.uk) and the North American CF Foundation (www.cff.org); all these organisations have published useful consensus and standards of care documents.